Transcript MUSCULAR DYSTROPHY - Unit2MuscleMadness

DISEASE ASSOCIATED
WITH MUSCLES IN
CHILDHOOD
MUSCULAR DYSTROPHY
WHAT IS MUSCULAR
DYSTROPHY?
Muscular Dystrophy (MD) is a disease in which the
muscles of the body get extremely weak and slowly
stop working.
 The genes in a persons body tell the body how to
make the proteins needed for the body to stay
healthy and strong. In MD sufferers these genes
give the body the wrong information which leads to
the proteins not being made properly.
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There are about 30 different forms of
Muscular Dystrophy which affect
children.
Some of these include:
 Duchenne Muscular Dystrophy
 Myotonic Dystrophy
 Limb-Girdle Muscular Dystrophy
 Facsioscapulohumeral Muscular Dystrophy
 Congenital Muscular Dystrophy
 Emery-Dreifuss Muscular Dystrophy
DUCHENNE MUSCULAR
DYSTROPHY
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The most common and severe type of MD is
Duchenne MD.
.It affects about one out of three and a half
thousand boys.
This disease usually affects boys because even
though girls can carry the gene, they usually have
no symptoms.
The symptoms usually appear at the young age of
about five when pelvic muscles start to weaken.
With other muscles in the shoulders, back, arms
and legs eventually also weakening. Most kids with
Duchenne MD need to use a wheelchair by the
age of about 12 and eventually a ventilator is
needed to assist breathing.
Duchenne MD occurs because of a problem with
the gene that creates a protein called dystrophin.
Without this protein the muscles break down and
weakness occurs
Kids with Duchenne MD usually have life span
of twenty years.
MYOTONIC DYSTROPHY
(STEINERT’S DISEASE)
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Myotonic Dystrophy is caused by a
portion of a particular gene being
larger than it should be.
Myotonic Dystrophy usually occurs
in 1 to 20,000 people and can
affect children as young as 10.
It usually starts in the muscles of
the hands, feet, neck and face.
The main symptoms are muscle
weakness, Myotona (when the
muscles have trouble relaxing after
they contract) and muscle wasting,
with the muscles wasting over time.
Children with myotonic dystrophy
can develop cataracts and have
heart problems.
LIMB-GIRDLE MUSCULAR
DYSTROPHY
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This type of MD equally effects both boys and
girls.
The symptoms usually begin to appear between
8 to 15 years old.
It progresses slowly and affects the pelvic,
shoulder and back muscles.
Muscle weakness varies with some children
having mild symptoms and others needing to
use a wheelchair when they are older.
FACIOSCAPULOHUMERAL
MUSCULAR DYSTROPHY
LANDOUZY-DEJERINE
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Facioscapulohumeral MD
usually develops in early
childhood and is noticeable
by the teen years.
It usually starts by affecting
the skeletal muscles of the
face, scapula and upper arms
and can affect other parts of
the body, for example it can
also affect the stomach
muscles, the biceps, triceps
and lower arm muscles.
CONGENITAL MUSCULAR
DYSTROPHY
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A person born with muscular dystrophy usually
has this type of MD.
People born with Congenital MD are usually
born with weak muscles and may have joint
deformities also.
This form of MD progresses fairly slowly.
EMERY-DREIFUSS MUSCULAR
DYSTROPHY
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Emery-Dreifuss MD usually affects the muscles used
for movement such as the skeletal muscles as well as
the cardiac muscles.
This disease usually affects people in early childhood
through to the teen years
The earliest features visible in people who have this
disease are joint deformities called contractures.
It mostly affects the elbows, ankles and neck.
Almost all people who suffer Emery-Dreifuss MD
usually also have heart problems which is noticeable by
adulthood.